ISSN: 1301-2193 E-ISSN: 1308-9846
  • Turkish Journal of
    Endocrinology and Metabolism
CASE REPORT

A Rare Combination: Multiple Endocrine Neoplasia Type 1 and Follicular Thyroid Carcinoma
Nadir Bir Kombinasyon: Multipl Endokrin Neoplazi Tip 1 ve Foliküler Tiroid Karsinomu
Received Date : 07 Jul 2019
Accepted Date : 02 Jan 2020
Available Online : 24 Jan 2020
Doi: 10.25179/tjem.2019-70603 - Makale Dili: EN
Turk J Endocrinol Metab 2020;24:87-91
Bu makale, CC BY-NC-SA altında lisanslanmış açık erişim bir makaledir.
ABSTRACT
Multiple endocrine neoplasia Type 1 (MEN-1) is an inherited syndrome characterized by the development of endocrine tumors of the pancreas, parathyroid, and pituitary glands. Mesenchymal tumors and adrenal neoplasms might also accompany this syndrome. However, the syndrome is rarely associated with thyroid tumors in contrary to the multiple endocrine neoplasia Type 2 that includes medullary thyroid carcinoma. This case study presents a 44-year-old woman who was diagnosed with MEN-1 on the basis of her clinical characteristics, laboratory data, and the presence of endocrine tumors. Follicular thyroid carcinoma was detected in the patient when she was being operated for nodular goiter, 12 years ago. We report this rare case which is likely the third case in the available scientific literature.
ÖZET
Multipl endokrin neoplazi Tip 1 (MEN-1) pankreas, paratiroid ve piuiter bezlerde endokrin tümörlerin gelişimiyle karakterize, kalıtımsal bir sendromdur. Mezenkimal tümörler ve adrenal neoplaziler de bu sendroma eşlik edebilir. Bununla birlikte, medüller tiroid karsinomu içeren multipl endokrin neoplazi Tip 2’nin aksine, bu sendrom, tiroid tümörleri ile nadiren ilişkilidir. Bu çalışmada, endokrin tümörlerin varlığı, laboratuvar verileri ve karakteristik klinik özelliklerin ışığında MEN-1 tanısı konan 44 yaşındaki bir kadın olgu sunulmaktadır. Olgu nodüler guatr için 12 yıl önce opere edildiğinde foliküler tiroid karsinomu tespit edilmişti. Mevcut bilimsel literatürde, muhtemelen 3. vaka olan bu ender olguyu bildirmekteyiz.
KAYNAKLAR
  1. Chandrasekharappa SC, Guru SC, Manickam P, Olufemi SE, Collins FS, Emmert-Buck MR, Debelenko LV, Zhuang Z, Lubensky IA, Liotta LA, Crabtree JS, Wang Y, Roe BA, Weisemann J, Boguski MS, Agarwal SK, Kester MB, Kim YS, Heppner C, Dong Q, Spiegel AM, Burns AL, Marx SJ. Positional cloning of the gene for multiple endocrine neoplasia-type 1. Science. 1997;276:404-407.
  2. Guo SS, Sawicki MP. Molecular and genetic mechanisms of tumorigenesis in multiple endocrine neoplasia type-1. Mol Endocrinol. 2001;15:1653-1664. [Crossref]  [PubMed] 
  3. Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx B, Falchetti A, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F, Wells SA Jr, Marx SJ. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001;86:5658-5671. [Crossref]  [PubMed] 
  4. Kim YL, Jang YW, Kim JT, Sung SA, Lee TS, Lee WM, Kim HJ. A rare case of primary hyperparathyroidism associated with primary aldosteronism, Hurthle cell thyroid cancer and meningioma. J Korean Med Sci. 2012;27:560-564. [Crossref]  [PubMed]  [PMC] 
  5. Lodewijk L, Bongers PJ, Kist JW, Conemans EB, de Laat JM, Pieterman CR, van der Horst-Schrivers AN, Jorna C, Hermus AR, Dekkers OM, de Herder WW, Drent ML, Bisschop PH, Havekes B, Rinkes IH, Vriens MR, Valk GD. Thyroid incidentalomas in patients with multiple endocrine neoplasia type 1. Eur J Endocrinol. 2015;172:337-342. [Crossref]  [PubMed] 
  6. Liaw CC, Lin JT, Chen TJ. Multiple-hormone-producing islet cell carcinoma: report of a case. Taiwan Yi Xue Hui Za Zhi. 1989;88:722-725.
  7. Thompson NW. Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the treatment of 40 patients with Zollinger-Ellison syndrome, hypoglycaemia or both. J Intern Med. 1998;243:495-500. [Crossref]  [PubMed] 
  8. Lodish MB, Powell AC, Abu-Asab M, Cochran C, Lenz P, Libutti SK, Pingpank JF, Tsokos M, Gorden P. Insulinoma and gastrinoma syndromes from a single intrapancreatic neuroendocrine tumor. J Clin Endocrinol Metab. 2008;93:1123-1128. [Crossref]  [PubMed]  [PMC] 
  9. Vortmeyer AO, Lubensky IA, Skarulis M, Li G, Moon YW, Park WS, Weil R, Barlow C, Spiegel AM, Marx SJ, Zhuang Z. Multiple endocrine neoplasia type 1: atypical presentation, clinical course, and genetic analysis of multiple tumors. Mod Pathol. 1999;12:919-924. /td>
  10. Perakakis N, Flohr F, Kayser G, Thomusch O, Parsons L, Billmann F, von Dobschuetz E, Rondot S, Seufert J, Laubner K. Multiple endocrine neoplasia type 1 associated with a new germline Men1 mutation in a family with atypical tumor phenotype. Hormones. 2016;15:113-117. [Crossref]  [PubMed]